Literature DB >> 15760880

Nervous system involvement in Degos disease.

Carmelo Amato1, Raffaele Ferri, Maurizio Elia, Filomena Cosentino, Carmelo Schepis, Maddalena Siragusa, Massimo Moschini.   

Abstract

Degos disease, or malignant atrophic papulosis, is a rare obstructive vasculopathy of unknown origin, characterized by distinctive skin lesions, visceral involvement, and an unfavorable outcome. The gastrointestinal tract and the central nervous system are most frequently affected, but cases limited to benign skin lesions have also been described. Neuroradiologic reports of this condition are exceptionally rare. We report the case of a 29-year-old woman with central and peripheral nervous system involvement who presented with progressive clinical deterioration and a meningovascular pattern at cerebral MR imaging; other organs were spared in this patient.

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Year:  2005        PMID: 15760880      PMCID: PMC7976473     

Source DB:  PubMed          Journal:  AJNR Am J Neuroradiol        ISSN: 0195-6108            Impact factor:   3.825


  13 in total

1.  Kohlmeier-Degos disease: a multisystem vasculopathy with progressive cerebral infarction.

Authors:  J N Burrow; P C Blumbergs; P V Iyer; J F Hallpike
Journal:  Aust N Z J Med       Date:  1991-02

2.  [Cerebral form of Degos's disease].

Authors:  P Warot; J C Caron; P Lehembre; M Houcke
Journal:  Rev Neurol (Paris)       Date:  1977-05       Impact factor: 2.607

Review 3.  The spectrum of neurologic disease associated with antiphospholipid antibodies. Lupus anticoagulants and anticardiolipin antibodies.

Authors:  S R Levine; K M Welch
Journal:  Arch Neurol       Date:  1987-08

4.  Malignant atrophic papulosis (Degos' disease) involving three generations of a family.

Authors:  S K Katz; L J Mudd; H H Roenigk
Journal:  J Am Acad Dermatol       Date:  1997-09       Impact factor: 11.527

5.  Degos' disease: association with anticardiolipin antibodies and the lupus anticoagulant.

Authors:  H J Englert; C H Hawkes; M L Boey; G J Derue; S Loizou; E N Harris; A E Gharavi; R G Hull; G R Hughes
Journal:  Br Med J (Clin Res Ed)       Date:  1984-09-08

6.  Degos' disease.

Authors:  F O Güven; K E Bozdağ; M Ermete; A Karaman
Journal:  Int J Dermatol       Date:  2000-05       Impact factor: 2.736

7.  Papulosis atrophicans maligna (Köhlmeier-Degos disease): a disseminated occlusive vasculopathy.

Authors:  H R McFarland; W G Wood; B V Drowns; A C Meneses
Journal:  Ann Neurol       Date:  1978-05       Impact factor: 10.422

Review 8.  Malignant atrophic papulosis.

Authors:  R Degos
Journal:  Br J Dermatol       Date:  1979-01       Impact factor: 9.302

9.  Benign Degos' disease developing during pregnancy and followed for 10 years.

Authors:  Thomas Bogenrieder; Marion Kuske; Michael Landthaler; Wilhelm Stolz
Journal:  Acta Derm Venereol       Date:  2002       Impact factor: 4.437

10.  CNS involvement in malignant atrophic papulosis (Kohlmeier-Degos disease): vasculopathy and coagulopathy.

Authors:  D K Dastur; B S Singhal; H J Shroff
Journal:  J Neurol Neurosurg Psychiatry       Date:  1981-02       Impact factor: 10.154

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  3 in total

Review 1.  Malignant atrophic papulosis with motor aphasia and intestinal perforation: A case report and review of published works.

Authors:  Pan Hu; Zhi Mao; Chao Liu; Xin Hu; Hongjun Kang; Feihu Zhou
Journal:  J Dermatol       Date:  2018-03-08       Impact factor: 4.005

2.  Stroke Classification: Critical Role of Unusually Large von Willebrand Factor Multimers and Tissue Factor on Clinical Phenotypes Based on Novel "Two-Path Unifying Theory" of Hemostasis.

Authors:  Jae C Chang
Journal:  Clin Appl Thromb Hemost       Date:  2020 Jan-Dec       Impact factor: 2.389

3.  Clinico-Pathological Features in Köhlmeier-Degos Disease with Cutaneous and Neurological Involvement.

Authors:  Dodmallur Mallikarjuna Sindhu; Sumanth Shivaram; Shilpa Rao; Madhu Nagappa; Doniparthi V Seshagiri; Vani Santosh; J Madhukara; A S Shreedhara; Maya D Bhat; Rose D Bharath; Sanjib Sinha
Journal:  Ann Indian Acad Neurol       Date:  2021-03-27       Impact factor: 1.383

  3 in total

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